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The main pulmonary artery (MPA) is seen, with the isolated innominate artery (IA) originating from the MPA. Note the long course of the IA prior to its division into the left common carotid artery and the left subclavian artery. AA = ascending aorta.

Origin of the Left Innominate Artery From the Main Pulmonary Artery in Two Dimensions and With Color Doppler Imaging

A 3-month-old patient with a known diagnosis of perimembranous ventricular septal defect and DiGeorge syndrome was admitted for an aspiration event. On admission, transthoracic echocardiography confirmed the known diagnosis of perimembranous ventricular septal defect. Suprasternal aortic arch imaging failed to demonstrate normal innominate artery origin. On careful evaluation, a right-sided aortic arch was noted, with isolated innominate artery originating from the main pulmonary artery (A, Online Videos 1 and 2). The right carotid and right subclavian arteries had normal origins from the aorta. Cardiac magnetic resonance imaging confirmed the diagnosis of isolated innominate artery from the main pulmonary artery (B to D).

Isolated innominate artery from the pulmonary artery is an exceedingly rare anomaly (1,2). There are only 2 previous reports of association with DiGeorge syndrome (3,4). Embryologically, the hypothetical double aortic arch of Edwards can explain the occurrence of isolated innominate artery (E) (5). Clinically, in the absence of an intracardiac shunt, differential cyanosis between the right and left arm should raise the suspicion for this anomaly. In most cases, including ours, the innominate artery can be relocated to the aorta with good results.

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